Table 1
Timeline of patient evolution.
| Time point | Clinical status | Key investigations | Management |
|---|---|---|---|
| Birth 2 years old | Pulmonary atresia, TGA, VSD Definitive repair | Echocardiography | Blalock-Taussig shunt Rastelli procedure and 14-mm Contegra RV-PA conduit implantation |
| 4 years old 8 years old | Recurrent conduit stenosis | Echocardiography Angio-CT Hemodynamic assessment by cardiac catheterization | Conduit replacement (16-mm Contegra) Conduit replacement (14-mm Contegra) + RPA stent implantation |
| ~2 years before admission (age 7) | Hepatomegaly, hypoalbuminemia, hypogammaglobulinemia, intermittent fever, recurrent hepatitis, splenomegaly | Liver biopsy: aggressive chronic hepatitis | Splenectomy |
| Admission (age 9) | Palpebral, scrotal and lower limb edema, ascites, hepatomegaly, reduced exercise tolerance | Echocardiography: severe RV-PA stenosis; angio-CT: conduit narrowing | Initial supportive therapy (diuretics, albumin, aspirin) |
| Age 9 | Hemodynamic assessment: cardiac catheterization severe (RV-PA) conduit stenosis, significantly elevated right ventricular systolic pressure, high transconduit gradient | Percutaneous transcatheter intervention: balloon dilation, pulmonary trunk stent, and Melody valve implantation | |
| Following months | Persistent fever, edema, reduced exercise tolerance | Echocardiography: rising RV-PA gradient | |
| Reoperation (age 11) | Persistent fever, edema, reduced exercise tolerance | Echocardiography: severe conduit stenosis | Hybrid surgery: conduit replacement (16-mm Contegra), PA bifurcation plasty, RPA balloon dilation |
| Immediate Postoperative period | Favorable hemodynamics | FISH positive for Coxiella burnetii; initial serology negative | Doxycycline and hydroxychloroquine |
| Three months follow-up | Clinical improvement; normalization of proteins and Ig levels | Seroconversion with high phase I and II IgG titers | Continued antimicrobial therapy |